Rosai-Dorfman disease presenting as extranodal renal mass

CASE REPORT

ROSAI-DORFMAN DISEASE PRESENTING AS EXTRANODAL RENAL MASS ANAND KRISHNAN, AZIZA NASSAR,

AND

PETER T. NIEH

ABSTRACT We present a unique case of a man presenting with an abnormal prostate and large renal mass who was ultimately found to have prostate cancer and renal Rosai-Dorfman disease. We discuss the method of diagnosis and treatment in this patient, as well as review the presentation, diagnosis, and treatment of this rare disease. UROLOGY 66: 1319.e17–1319.e19, 2005. © 2005 Elsevier Inc.

R

osai-Dorfman disease is a rare disease of unknown etiology with limited reports in urologic journals. We report a case of a man with an abnormal digital rectal examination and a renal mass who proved to have prostate cancer and renal Rosai-Dorfman disease. We review the clinical presentation and essential histopathologic features necessary for the accurate diagnosis of this disease. CASE REPORT A 76-year-old white man was referred by his primary care physician for an abnormal prostate examination with a serum prostate-specific antigen level of 2.7 ng/mL. His past medical history revealed no pertinent medical conditions or family history of prostate cancer. The physical examination was only significant for a 45-g diffusely nodular prostate. He underwent transrectal ultrasoundguided sextant prostate needle biopsy, with the finding of Gleason score 5 ⫹ 4 ⫽ 9 adenocarcinoma of the prostate in six of six cores. In anticipation of external beam radiotherapy, he received androgen deprivation therapy. He also underwent a staging bone scan, which was negative for metastatic disease, and an abdominal and pelvic CT scan, which revealed an incidental 9-cm centrally located left renal mass (Fig. 1), demonstrating heterogeneous contrast enhancement. Positron emission tomography with 18F-fluoroaminocyclopentane From the Departments of Urology and Pathology, Emory University School of Medicine, Atlanta, Georgia Address for correspondence: Peter T. Nieh, M.D., Department of Urology, Emory University School of Medicine, 1365 Clifton Road NE, Building B, Atlanta, GA 30322. E-mail: peter.nieh@ emoryhealthcare.org Submitted: March 29, 2005, accepted (with revisions): June 22, 2005 © 2005 ELSEVIER INC. ALL RIGHTS RESERVED

carboxylic acid, an investigational radiotracer with minimal renal and bladder excretion, showed mild uptake in the renal mass, intense uptake in the paraaortic, paracaval, and retrocaval lymph nodes, and less intense uptake in a few pelvic nodes. With a presumptive diagnosis of synchronous adenocarcinoma of the prostate and either renal cell or transitional cell carcinoma of the left kidney with hilar nodal metastases, he underwent laparoscopic hand-assisted left radical nephrectomy with regional lymph node dissection. The operation was performed without complications. The final pathologic examination of the renal mass showed Rosai-Dorfman disease (Fig. 2), and the regional lymph node dissection revealed Gleason score 5 ⫹ 4 ⫽ 9 adenocarcinoma of the prostate. The patient underwent complete androgen deprivation for the prostate cancer. After withdrawal of the antiandrogen, he was asymptomatic with a prostate-specific antigen level of 2.6 ng/mL 1 year later. COMMENT The presence of prostate cancer in the renal hilar nodes was most surprising, but it highlights the aggressiveness of Gleason score 9 disease. The point of this discussion is the unusual finding in the enlarged left kidney. Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman.1 It is classified as a non-neoplastic histiocytic disorder and is characterized by massive lymphadenopathy secondary to infiltration and dilation of the lymph node sinuses by large histiocytes. It usually presents in younger individuals, in the first or second decade, with a slightly increased prevalence in African Americans and males. It classically presents with chronic, bilateral, nontender cervical lymphad0090-4295/05/$30.00 doi:10.1016/j.urology.2005.06.103 1319.e17

FIGURE 1. Computed tomography scan (A) without contrast showing large 9-cm solid left renal mass that enhanced (B) with contrast.

FIGURE 2. (A) High-power magnification demonstrating histiocytic proliferation with intracytoplasmic red blood cells, plasma cells, and lymphocytes—emperipolesis (arrowhead). Hemosiderin deposition due to digested red blood cells also noted (asterisk). Hematoxylin-eosin stain, original magnification ⫻60). (B) High-power magnification with cytoplasmic immunostaining of histiocytic marker (CD68) highlighting proliferating cells (arrowheads) (CD68/KPI stain, original magnification ⫻60).

enopathy of massive proportions. Extranodal disease has been reported in 33% to 40% of cases2– 4 and is less common than the classic nodal variant. Approximately 4% of all cases of Rosai-Dorfman disease are associated with kidney involvement, which, in some cases, represents the sole manifestation of the disease.5 Only one other case has been reported of renal Rosai-Dorfman disease in a patient with a prior history of the classic nodal variant later presenting with bilateral renal masses.2 Histologically, the sine qua non of Rosai-Dorfman disease is emperipolesis, in which histiocytes with eosinophilic cytoplasm can be seen engulfing lymphocytes without digesting them. The less common extranodal variants of the disease tend to occur more often in older patients and histologically may show less emperipolesis and more fibrosis. This may make the diagnosis more difficult, unless a high de1319.e18

gree of clinical suspicion is present. Immunohistochemistry will typically show positive staining of histiocytes for S-100 in almost all cases, as well as positive staining for other antigens specific to the macrophage lineage or activated macrophages, such as CD68, CD14, Ki-M1P, MRP-8, and MRP14. Histiocytes are characteristically negative for CD1a staining.2,6 Clinically, most patients will have an indolent course, with spontaneous resolution of symptoms, but persistent stable disease. Fatalities, although uncommon, have been reported in up to 7% of cases, although usually these have been due to underlying comorbid conditions.7 Chemotherapy and radiotherapy tend to have a variable response, although their use, as well as corticosteroid use, has been advocated in case reports, with a good response. Surgery is reserved only for those with UROLOGY 66 (6), 2005

compromise of adjacent vital organs by the disease. Positron emission tomography has been reported to show positive uptake in proven Rosai-Dorfman disease and may be a useful adjunct to the diagnosis, as was the case in our patient.8 The etiology is unknown, although an infectious or chronic inflammatory cause has been implicated. It is also known that clinically significant immunologically mediated disease can occur in association with sinus histiocytosis with massive lymphadenopathy and may adversely affect the prognosis.5 To our knowledge, this represents the first reported case of extranodal variant Rosai-Dorfman disease presenting as a renal mass in an elderly patient without a prior history of the nodal variant disease. REFERENCES 1. Rosai J, and Dorfman RF: Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathologic entity. Arch Pathol 87: 63–70, 1969.

UROLOGY 66 (6), 2005

2. Kugler A, Middel P, Gross AJ, et al: Unusual bilateral renal histiocytosis: extranodal variant of Rosai-Dorfman disease. J Urol 157: 942–944, 1997. 3. Gilbert-Barness E: Pathological case of the month. Arch Pediatr Adolesc Med 149: 57–58, 1995. 4. Rodriguez-Galindo C, Helton KJ, Sanchez ND, et al: Extranodal Rosai-Dorfman disease in children. J Pediatr Hematol Oncol 26: 19 –24, 2004. 5. Foucar E, Rosai J, and Dorfman R: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 7: 19 –73, 1990. 6. Lu D, Estalilla OC, Manning JT Jr, et al: Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma involving the same lymph node: a report of four cases and review of the literature. Mod Pathol 13: 414 – 419, 2000. 7. Foucar E, Rosai J, and Dorfman RF: Sinus histiocytosis with massive lymphadenopathy: an analysis of 14 deaths occurring in a patient registry. Cancer 54: 1834 –1840, 1984. 8. Yu JQ, Zhuang H, Xiu Y, et al: Demonstration of increased FDG activity in Rosai-Dorfman disease on positron emission tomography. Clin Nuclear Med 29: 209 –210, 2004.

1319.e19